Temperature intolerance is a common and often debilitating symptom of postural tachycardia syndrome (POTS), however treatment options are limited. In this open-label pilot study, we examined the use of a novel wearable thermal watch (Embr Wave2™) on thermoregulatory and other autonomic symptoms in POTS. Participants with POTS and temperature intolerance were recruited from the Stanford autonomic disorders clinic. All patients completed an online battery of autonomic and sleep questionnaires including the composite autonomic symptom score-31 (COMPASS-31), the Delphi interoceptive scale, the temperature disturbance index scale (TDIS), and a 10-minute active stand testing at baseline and after 4 weeks of wearable use. Twenty-two POTS participants with severe temperature intolerance were included in the final analysis. At baseline, 55% noted a moderate to severe impact of temperature intolerance on quality of life (QoL). After 4 weeks of wearable use, sustained reductions were seen across multiple TDIS domains, including work, leisure activities, and enjoyment of life (p ≤0.05). No significant changes were seen in COMPASS-31 scores (53.98 [43.36-56.62] vs. 53.04 [44.66-60.30], p = 0.34) or orthostatic tachycardia on stand testing (31.5 [24.8-45.5] bpm vs.31.0 [21.0-40.5] bpm, p = 0.56). Temperature intolerance is common and directly correlated with QoL in patients with POTS. Use of a non-invasive thermal wearable led to improvement in temperature related QoL measures, however global autonomic symptom scores and orthostatic tachycardia remained unchanged. This study highlights the need for other treatment studies with more specific patient reported outcomes measures in POTS patients with temperature intolerance.

Cardiac autonomic dysfunction is a well-established sequela of concussions, most commonly manifesting in the postconcussive period. Athletes are typically susceptible to these forms of injury. This article summarizes the scope of existing research on this topic, providing evidence and clinical experience of the evaluation, diagnostic modalities, differential diagnoses, and treatment considerations from the sports cardiology perspective.

Patients with postural orthostatic tachycardia syndrome (POTS) frequently report higher rates of chronic gastrointestinal symptoms, disorders of gut-brain interaction (DGBI), and extra-intestinal co-morbidities. We conducted a systematic review and meta-analysis to assess the prevalence of gastrointestinal symptoms and comorbid conditions in POTS patients. Electronic databases were searched from inception until May 2025 for studies reporting gastrointestinal symptoms in POTS patients. A random-effects model was used to pool the proportion of POTS patients reporting gastrointestinal symptoms, and sub-group analyses were conducted. The final dataset includes 19 studies, with 8268 POTS patients, revealing that 57.9% (95% CI 38.4-75.2) had at least one gastrointestinal symptom. The most common gastrointestinal symptom was nausea (70.1%, 95% CI 51.5-83.7) followed by bloating (64.9%, 95% CI 48.5-78.4), abdominal pain (60.4%, 95% CI 39.2-78.3) and postprandial fullness (60.4%, 95% CI 45.6-73.6). Irritable bowel syndrome was the most prevalent DGBI, affecting 26.8% (95% CI 15.3-42.4) of POTS patients. The most common extraintestinal comorbidity was anxiety, reported in 42.9% (95% CI 22.7-65.8), followed by chronic fatigue (40.9%, 95% CI 21.1-64.2), migraine (35.6%, 95% CI 27.0-45.2), depression (34.4%, 95% CI 19.0-54.0), and fibromyalgia (21.6%, 95% CI 12.8-34.2). Approximately one third reported mast cell activation syndrome (36.3%, 95% CI 17.8-60.0) and joint hypermobility syndrome (31%, 95% CI 24.4-38.5). There was substantial heterogeneity seen in the primary and most subgroup analyses. Overall, 60% of POTS patients report concurrent gastrointestinal symptoms, with nausea being the most common. IBS affects 25% of patients with POTS. Notably, extra-intestinal comorbidities-primarily anxiety, chronic fatigue, migraines, depression, and fibromyalgia-are more prevalent than gastrointestinal conditions in this population.

More than five years after the start of the COVID-19 pandemic, its long-term effects are increasingly coming into focus. Post-COVID disease poses a significant challenge, - not only for the individuals affected, but also for healthcare providers and society as a whole. In order to improve care for post-COVID patients, the current state of research should be reviewed and the frequent and characteristic complaints post-exertional malaise and postural tachycardia syndrome should be presented. The literature search for this narrative review was conducted in the PubMed and Semantic Scholar databases. Post-COVID symptoms are often nonspecific, diverse, and fluctuating. However, post-exertional malaise and postural tachycardia syndrome are characteristic of post-COVID when they occur newly after COVID-19 disease. Post-exertional malaise is an intensification of symptoms after exertion that occurs in about 86% of post-COVID patients. Pacing is a promising treatment approach here. Postural tachycardia syndrome manifests as autonomic, tachycardic, orthostatic dysregulation and affects up to 82% of post-COVID patients. Symptomatic therapy includes pharmacological and non-pharmacological measures. Post-exertional malaise and postural tachycardia syndrome are typical and characteristic post-COVID symptoms. Current scientific findings underscore the SARS-CoV-2-related organic origin of post-COVID symptoms.

Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) and Long COVID (LC) are complex multisystem conditions with significant functional disability. Many patients experience symptoms of orthostatic intolerance, which can be captured in some cases as Orthostatic Hypotension (OH) or Postural orthostatic Tachycardia Syndrome (PoTS) on objective testing. Conservative treatments are recommended for first-line symptom management, but there is a lack of efficacy evidence. This study aims to assess the feasibility of an 8-week clinically supervised, personalised Dysautonomia Management Protocol (DMP) in a cohort of ME/CFS and LC patients with subjective and objective evidence of orthostatic intolerance (dysautonomia). ME/CFS and LC patients with objective dysautonomia on the 10 min active Lean Test (LT) were recruited to an 8-week DMP, with interventions introduced cumulatively every two weeks. Interventions included increasing daily fluid intake to 3 litres and salt intake to 10 g, pacing to avoid crashes and calf activation. Baseline and weekly data collection included the LT, Composite Autonomic Symptom Score questionnaire (COMPASS-31) and Yorkshire Rehabilitation Scale (YRS). Sixteen participants completed the 8-week program, five discontinued during the program, and one was withdrawn following a severe crash. The COMPASS-31 improved by 7.7 points from week 1 to week 8 ( = 0.045), with a medium Cohen's d effect size of 0.55. For the same period, there was a non-significant ( = 0.16) improvement in the YRS symptom severity score by 2 points. Comparing the final two weeks of the program with the first two weeks, mean heart rate during the LT decreased by 4.8 beats per minute ( = 0.032), with a medium Cohen's d effect size of 0.44. Adherence to the interventions was highly variable, with none of the patients able to fully employ all four recommendations. The results suggest that targeted conservative interventions could influence autonomic function and symptom reduction. However, the magnitude of change was limited, and statistical significance might not necessarily relate to a clinically significant improvement in symptoms.

Vascular compression syndromes are increasingly recognized as underdiagnosed contributors to morbidity in patients exhibiting dysautonomia. Underlying vascular compression syndromes affecting the head and neck, abdomen, pelvis, and lower extremities may influence venous return, neurohormonal signaling, and autonomic regulation. There is considerable clinical overlap among these syndromes, as well as between hypermobility spectrum disorders (HSD) and dysautonomia, indicating possible shared or interacting pathophysiological mechanisms. This hypothesis-generating narrative review synthesizes current evidence linking vascular compression syndromes with dysautonomia, highlights potential mechanistic pathways, identifies patterns of syndromic overlap, and emphasizes the importance of systematic evaluation in affected patient populations. Evidence from retrospective studies, case series, and clinical observations indicates that vascular compression syndromes may be prevalent among patients with dysautonomia, particularly postural orthostatic tachycardia syndrome (POTS) and HSD, yet are often unrecognized. Proposed mechanisms based on limited data include impaired venous capacitance and preload reserve, increased intracranial pressure, altered renin-aldosterone and cortisol signaling, underlying autoimmune and systemic diseases, and sympathetic ganglion irritation. Several compression syndromes show symptom overlap and frequent co-occurrence, especially in patients with connective tissue disorders. Emerging data suggest that targeted interventions, such as surgical decompression or venous stenting, may improve orthostatic intolerance and quality-of-life measures in selected patients, though high-quality prospective data remain limited. Vascular compression syndromes may be an important yet underappreciated contributor to dysautonomia. Increased clinical awareness and systematic screening may reduce diagnostic delays and morbidity in this underserved population. Prospective studies are needed to clarify prevalence, establish causal relationships, and determine the impact of targeted treatments on autonomic outcomes.

Orthostatic intolerance (OI) is an important factor affecting daily functional capacity in patients with long COVID. Traditionally, most OI symptoms have been attributed to exaggerated sympathetic nervous system activation associated with postural orthostatic tachycardia syndrome (POTS). Disequilibrium, also referred to as postural instability, may contribute to the development of OI in patients with long COVID. This study evaluated 32 patients with long COVID using neurological examinations and the active 10-min standing test. Disequilibrium was assessed using the Romberg and tandem gait tests. OI was defined as the inability to complete the active 10-min standing test. Seven patients (22%) were diagnosed with OI. None of them had POTS, whereas six (86%) demonstrated disequilibrium, as detected by the Romberg and/or tandem gait test. POTS was observed in eight patients (25%), none of whom had OI. Disequilibrium was observed in nine patients (28%), six of whom (67%) had OI. Multiple regression analysis revealed that disequilibrium was positively associated with OI (r = 0.64, < 0.001), whereas POTS was inversely associated (r = -0.38, < 0.05). After 6 weeks of oral minocycline treatment in six patients and 2 weeks of repetitive transcranial magnetic stimulation therapy following minocycline in the other one patient, symptom amelioration was reported in six patients with OI. OI concomitant with disequilibrium recovered in five of the six patients treated and tested, although one patient who experienced symptom recovery failed to undergo the repeated standing test. Disequilibrium, rather than POTS, was the primary determinant of OI in patients with long COVID.

Patients with Ehlers-Danlos Syndrome (EDS) and hypermobility spectrum disorder (HSD) often complain of palpitations, presyncope/syncope, attributable to postural orthostatic tachycardia syndrome (POTS). Occasionally, the etiology of these complaints is not positional, unclear, and may require further cardiac evaluation, including cardiac monitoring. To our knowledge, the utility of implantable loop recorders (ILRs) has yet to be explored in this population. This study aimed to evaluate the utility of the ILRs in diagnosing and/or excluding arrhythmias not attributable to POTS in symptomatic patients with EDS and HSD. Patients with EDS and HSD were referred from the New York Institute of Technology (NYIT) Ehlers-Danlos Syndrome/Hypermobility Treatment Center to the Long Island Heart Rhythm Center (LIHRC) for cardiac evaluation between January 2019 and November 2023. A retrospective analysis of observational de-identified data from the LIHRC was permitted by the NYIT College of Osteopathic Medicine Institutional Review Board (BHS-1465). Patients were monitored monthly, and the utility of the ILRs was assessed based on a correlation of symptoms to an arrhythmia, the ability of the device to monitor therapy efficacy, and/or to rule out an arrhythmia as the explanation of symptoms. Data is reported as percentages and mean ± standard deviation (SD). A total of 116 hypermobile patients (81 EDS/35 HSD) were evaluated. Among these patients, 31 (26.7 %) received an ILR (length of follow-up, 27.3 months ± 14.0 months), 29 females/2 males, 28 patients had EDS, 3 patients had HSD, age 34.1 ± 11.4 years. Symptomatic sinus tachycardia (ST) occurred in 16 patients (51.6 %), and ST helped diagnose POTS and/or monitor therapy in 15 (48.4 %). Symptomatic premature ventricular complexes (PVCs) occurred in 8 patients (25.8 %), supraventricular tachycardia (SVT) in 6 (19.4 %), and ventricular tachycardia (VT) in 1 (3.2 %). In 2 patients, ILR findings led to further interventions, including PVC ablation and an implantable cardioverter-defibrillator (ICD) for symptomatic VT. The ILR demonstrated utility in all patients. This study demonstrated the utility of the ILR in identifying symptomatic arrhythmias in patients with EDS and HSD. ILR monitoring also aided in solidifying a POTS diagnosis and guiding patient management/treatment efficacy. Further evaluation in a larger cohort is needed to further understand the impact of ILR monitoring in hypermobile patients.

A 39-year-old woman developed debilitating systemic symptoms following the placement of a synthetic mid-urethral sling for stress urinary incontinence. Previously in good health, she experienced sepsis and adrenal crisis immediately postoperatively, followed by persistent symptoms including fatigue, postural orthostatic tachycardia syndrome (POTS), neuropathic pain, cognitive dysfunction, autonomic instability, and recurrent infections. Despite extensive multispecialty evaluations, no definitive diagnosis was initially reached. Laboratory testing later revealed positive ANA and mild immune dysregulation. Given her complex autoimmune history, strong family predisposition, and the temporal association with mesh implantation, her presentation was suspected to be consistent with autoimmune syndrome induced by adjuvants (ASIA). She elected to undergo surgical removal of the synthetic sling with placement of an autologous rectus fascia pubovaginal sling. Postoperatively, the patient experienced significant resolution of her symptoms, restored bladder function, and improved quality of life. This case demonstrates a temporal association consistent with ASIA, with complete symptom resolution by 3 months post-explant and sustained well-being at 1 year.

Syncope is a manifestation of autonomic dysfunction after high spinal cord injury. However, it is rarely reported as a feature of postural orthostatic tachycardia syndrome (POTS) after spinal cord injury. This case report describes a male in his 50s suffering from C2 spinal cord injury who developed recurrent postural syncope post-injury. These events were characterized by orthostatic tachycardia upon standing and could even be induced by seated head-tilt maneuvers, fulfilling the diagnostic criteria for POTS. These patients have substantial risks of fall-related morbidity. Heart rate variability and sympathetic skin response assessments help elucidate the underlying autonomic pathophysiological mechanisms. Syncope may be the predominant symptom during transfers, standing, or seated head-tilt positioning. Notably, documenting heart rate fluctuations during transient syncopal events is challenging. Infections constitute established triggers for syncope. Comprehensive management strategies may achieve complete resolution of syncopal episodes.

Syncope is an abrupt, transient, and complete loss of consciousness associated with an inability to maintain postural tone, followed by rapid and spontaneous recovery. Syncope is caused by temporary cerebral hypoperfusion. Presyncope describes symptoms such as lightheadedness and vision changes that may or may not precede syncope. Syncope can be classified by its mechanism: reflex (neurally mediated), orthostatic hypotension, or cardiac. Initial evaluation of loss of consciousness should include a careful history and detailed physical examination with orthostatic vital signs and 12-lead electrocardiography. The history should evaluate whether the episode was a true loss of consciousness and clarify whether it has a syncopal or nonsyncopal etiology. Although there is no evidence-based standard for the diagnosis of syncope, consensus suggests diagnostic criteria concordant with the three syncope mechanisms. The treatment of syncope is specific to its mechanism. Multiple risk stratification tools exist; however, these do not outperform clinician judgment in predicting serious outcomes of syncope in the short term. When evaluating and treating syncope, additional consideration should be given to special populations including children and adolescents, older adults, athletes, and patients with postural orthostatic tachycardia syndrome.

Hyperadrenergic postural orthostatic tachycardia syndrome (hPOTS) is a subgroup of postural orthostatic tachycardia syndrome with prominent sympathetic activation symptoms. We recently recorded skin sympathetic nerve activity (SKNA) in ambulatory participants with hPOTS at baseline and after being treated with clonidine.1 We performed a secondary analysis to determine whether reduced average SKNA (aSKNA) during sleep was associated with ST-segment elevation. Key Findings: There is a negative correlation between average skin sympathetic nerve activity (aSKNA) and the septal ST-segment height; there is a higher septal ST segment and lower aSKNA when asleep compared with awake; reduced aSKNA at night is associated with nocturnal ST-segment elevation.

Long COVID presents with a variety of symptoms, some of which could be related to autonomic dysfunction. Our aim was to evaluate the prevalence of autonomic dysfunction in long COVID patients. We conducted a cross-sectional study and included all consecutive patients enrolled in several clinical research studies. We performed the following autonomic dysfunction markers: heart rate variability, heart rate, systolic and diastolic blood pressure changes during NASA Lean Test, cardiopulmonary exercise testing and a Composite-Autonomic-Symptom-Score (COMPASS)-31 scale. We used linear regression to calculate the contribution of each dysautonomia measure on symptom burden as measured by the modified COVID-19 Yorkshire scale. We included 100 patients for this study. Our sample population had a mean age of 56+/-11 years, included 53% minorities, and 32% were women. Dysautonomia, as defined by an abnormal COMPASS-31, was seen in 82% (95% confidence interval [CI] 72-89) of our study population, while cardiovascular resting dysautonomia, as represented by an abnormal heart rate variability, was seen in 60% (95% CI 48-70) of our study population. Orthostatic hypotension was observed in 12% of our study population, and postural orthostatic tachycardia syndrome (POTS) was found in 10% of our study population. In our adjusted analysis, we found that the beta coefficient for the COMPASS-31 score (0.37) was significant on changes in a self-reported long COVID symptom burden. The orthostatic intolerance and gastrointestinal domains of the COMPASS-31 were associated with the highest long COVID symptom burden. Dysautonomia is common in long COVID patients and contributes to the overall symptoms seen in long COVID. Identifying dysautonomia has important diagnostic and therapeutic implications.

Postural orthostatic tachycardia syndrome (POTS) is characterized by orthostatic tachycardia with associated symptoms including presyncope, fatigue, dizziness, and gastrointestinal complaints, among others. In addition to cardiovascular and neurologic features, autonomic dysfunction may involve other organ systems. We report a rare case of transient evanescent hyperemia occurring during presyncopal episodes in a patient with POTS, highlighting a potentially underrecognized dermatologic sign of autonomic dysfunction. We present a 19-year-old female with known POTS and a complex medical history including pituitary adenoma (prolactinoma), secondary adrenal insufficiency, gastroparesis, severe malnutrition, and pelvic floor dysfunction, who was admitted for recurrent presyncope, syncope, and collapse. Her presentation was multifactorial. Contributing factors included autonomic instability, cabergoline-related effects, and nutritional compromise. During hospitalization, she developed recurrent episodes of transient, sharply demarcated erythematous patches affecting the face, chest, and upper extremities that coincided with presyncope and resolved spontaneously without intervention. Dermatology evaluation supported a diagnosis of evanescent hyperemia in the setting of autonomic dysfunction associated with POTS. Diagnostic workup included serial laboratory testing, electrocardiography, echocardiography, neuroimaging, and multidisciplinary specialty consultations. Transthoracic echocardiography demonstrated a patent foramen ovale with preserved cardiac function, without evidence of structural heart disease contributing to her symptoms. Management required a multidisciplinary approach, including stress-dose intravenous hydrocortisone for adrenal insufficiency, adjustment of cabergoline due to suspected medication-related bradycardia, continuation of fludrocortisone for volume support, and initiation of nasoduodenal tube feeding for nutritional rehabilitation. This case illustrates a transient cutaneous finding temporally associated with presyncope in a patient with POTS and complex comorbidities. Awareness of such skin changes during symptomatic episodes may provide supportive clinical clues to underlying autonomic dysfunction, particularly in diagnostically challenging presentations.

Ehlers Danlos and related hypermobility syndromes underly chronic, recurrent, and multiple-site pain in a small number of persons. By making the diagnosis, clinicians can better guide the treatment of the presenting problem. The diagnosis can help a patient make sense of their recurrent challenges as well. By inquiring about numerous associated conditions, ranging from postural orthostatic tachycardia syndrome to autism spectrum and attention deficit issues, the clinician may help the patient deal with other challenges. Detection can be easy-primarily observation of flexibility in the thumb, finger, elbow, and knee. The diagnosis is missed commonly, so we recommend clinicians take 1 month to perform these maneuvers on all chronic pain patients as one way of ensuring that clinicians have awareness.

Autonomic dysfunction, particularly postural orthostatic tachycardia syndrome (POTS), is increasingly recognized in adolescents and young adults. Early recognition in primary care is critical, as these conditions can substantially affect functioning, school participation, quality of life, and health care utilization. This review provides practical, clinically relevant guidance for pediatric and family medicine clinicians, and emphasizes recognition, office-based assessment, initial management, and referral strategies. Key considerations include differentiating POTS from mimicking conditions, evaluating functional impact, and implementing structured lifestyle interventions. Medications are reserved for patients with significant functional impairment despite conservative management, with a focus on setting realistic expectations. Case vignettes illustrate common phenotypes and highlight practical approaches to individualized care. By providing clear frameworks for evaluation and management, primary care clinicians can reduce unnecessary specialty visits, improve patient outcomes, and facilitate coordinated care across multidisciplinary teams.

How is postural orthostatic tachycardia syndrome (POTS) diagnosed? What about adolescents who seem to have POTS but do not meet the diagnostic criteria? How can we treat POTS and related conditions? How can we best respond to common questions of frustrated patients and parents and guardians? This article provides evidence- and expert-based answers to questions that frequently arise when caring for patients with POTS and related conditions.

β-thalassemia minor is associated with high rates of anaemia developing in pregnancy, possibly related to a blunted erythropoietin response. Anaemia with an inadequate erythropoietin response has been demonstrated in non-pregnant individuals with autonomic neuropathy with normal renal function. A case of mild anaemia in pregnancy with severe orthostatic symptoms in a woman with β-thalassemia minor and possible postural orthostatic tachycardia syndrome, with rapid resolution of symptoms and improvement in anaemia with darbepoetin therapy is described. The potential role of erythropoietin-stimulating agents in the management of symptoms in pregnancy-related anaemia in thalassemia minor is discussed.

To investigate the changes in T-wave amplitude and Tp-Te interval on supine and standing electrocardiograms (ECGs) in pediatric postural orthostatic tachycardia syndrome (POTS), and to explore their predictive value for the therapeutic effect of metoprolol. A total of 59 children diagnosed with POTS who presented with syncope or pre-syncopal symptoms were enrolled as the POTS group, and 52 healthy children served as the control group. Supine and standing ECGs were recorded for all subjects, and T-wave amplitude and Tp-Te interval were measured. Children with POTS were followed-up after metoprolol treatment and divided into a therapeutic response group and a non-response group. (1) Comparison of supine vs. standing ECGs: In the POTS group, standing posture (compared with supine posture) was associated with increased heart rate (HR), decreased T-wave amplitude in leads II, III, aVF, V4, V5, and V6, shortened Tp-Te interval in leads I, II, III, aVR, aVF, V1, V3, V4, V5, and V6, and elevated Tp-Te/QT ratio in leads aVL and V5 (all < 0.05). (2) Comparison with the control group: The POTS group exhibited a greater HR difference (ΔHR), as well as larger differences in T-wave amplitude (ΔT-wave amplitude) between supine and standing positions in leads II, aVR, aVL, aVF, V3, and V5 (all < 0.05). (3) Follow-up: Compared with the non-response group, the therapeutic response group showed larger ΔT-wave amplitude in leads III, aVF, V2, V3, V4, and V5, larger Tp-Te interval difference (ΔTp-Te interval) in lead V3, and larger Tp-Te/QT ratio difference (ΔTp-Te/QT ratio) in lead V3 (all < 0.05). (4) Receiver operating characteristic curve: ΔT-wave amplitude in leads III, aVF, V2, V3, V4, and V5, ΔTp-Te interval in lead V3, and ΔTp-Te/QT ratio in lead V3 all had predictive value for the therapeutic effect of metoprolol in pediatric POTS (all < 0.05). ΔHR and ΔT-wave amplitude in lead V5 between supine and standing positions are independent risk factors for pediatric POTS. A combination of five indicators-ΔT-wave amplitude in leads V2, V3, and V5, ΔTp-Te interval in lead V3, and ΔTp-Te/QT ratio in lead V3 between supine and standing ECGs-exerts a good predictive effect on the therapeutic response of pediatric POTS to metoprolol intervention.

: Difficulty swallowing is a common complaint in patients with postural orthostatic tachycardia syndrome (POTS), but there are no qualitative studies that examine dysphagia in patients with POTS, resulting in a significant gap in clinical understanding and research. : A structured interview of patients with autonomic disorders was conducted utilizing the Dysphagia Handicap Index (DHI). : Eleven participants (age range 21-71, mean age 46 years, eight women) were selected using purposive sampling through online support communities and referrals from Dysautonomia Clinic. All had POTS, and eight had comorbid Ehlers-Danlos syndrome. The data gathered from participants were used to construct thematic descriptions of their lived experiences. The mean DHI score in this cohort was 4.5, indicating significant impairment in swallowing. Four themes emerged from the participant narratives: (1) the negative physical impact of dysphagia, (2) the negative psychological impact of dysphagia, (3) the impact on daily life and relationships, and (4) reduced healthcare satisfaction. : We found significant impairment due to reported dysphagia symptoms in patients with POTS. Further studies are needed to elucidate the pathophysiology, severity and type of dysphagia in POTS and to develop targeted therapies.

Joint Hypermobility Syndrome (JHS), also referred to as hypermobile Ehlers-Danlos Syndrome (hEDS), is increasingly recognised as a significant contributor to gynaecological, pelvic floor and lower urinary tract symptoms. This review aims to highlight the current evidence on the relationship between JHS and key urogynaecological conditions, with particular attention to recent developments in epidemiology, clinical presentation, and management. Emerging research demonstrates a strong association between JHS and pelvic organ prolapse (POP), with meta-analytic data showing more than a twofold increased risk. Large cohort studies highlight substantial symptom burden, including high rates of stress and urgency incontinence, bladder pain, pelvic floor dysfunction and sexual difficulties. Recent work also reveals distinct challenges in perioperative care, such as dysautonomia-related anaesthetic risks, tissue fragility, impaired wound healing and higher rates of surgical complications in some series. Studies document higher prevalence of urinary incontinence, bladder diverticula, recurrent urinary infections and voiding dysfunction linked to increased bladder capacity and elevated residual volumes. Women with JHS experience a wide and often severe spectrum of urogynaecological symptoms that significantly affect quality of life. Although management generally mirrors standard care pathways, the unique connective-tissue and systemic features of JHS necessitate tailored assessment and multidisciplinary involvement. Further high-quality research is required to develop specific management algorithms and clarify the role of conservative versus surgical treatments in this complex clinical population.

: Postural orthostatic tachycardia syndrome (POTS) includes multiple symptoms and comorbid conditions. Assessment for less recognized symptoms and conditions was performed through a telemedicine-only clinic for adolescent and young adult patients with POTS. : A retrospective review of records was performed for information obtained during clinical care. Patients up through the age of 23 years were evaluated, either diagnosing or confirming a diagnosis of POTS, and identifying other symptoms and diagnoses. These data were evaluated for differences, including by sex and presence or absence of joint hypermobility. : In total, 277 patients met the inclusion criteria. The median age was 16.8 years (IQR 15.2-19.1); 88.1% were female. Suspected mast cell activation syndrome occurred in 70% of patients. Joint hypermobility was found in 78.3% of patients; female patients were more affected (80.3% versus 63.6%); 57.0% had both suspected MCAS and joint hypermobility. Migraine was seen in 51.6% of patients; 57.4% had tension-type headache. Females appeared more likely to have tension headache or both types of headache together, while males seemed more likely to have migraine. Joint hypermobility did not influence headache presence or absence. A history of head trauma/concussion was reported in 39.7% of patients, with 14.4% having vestibular symptoms and 4% having convergence disorder. Without head trauma/concussion, 23.8% of patients reported vestibular symptoms, convergence disorder, or both. : We report previously unrecognized or poorly described symptoms and conditions accompanying POTS. Recognition of these symptoms and conditions in patients with POTS can allow for more complete evaluation and management of debilitating factors and may give insights into underlying pathophysiologies leading to POTS.

The objective of this study was to investigate the potential association between the use of calcitonin gene-related peptide (CGRP) antagonists and the reporting of cardiac adverse events (cAEs) by analyzing data from the US Food and Drug Administration Adverse Event Reporting System (FAERS). CGRP antagonists are a novel class of effective treatments for migraine. However, given CGRP's crucial role as a potent vasodilator, concerns about the cardiac safety of its long-term blockade persist. This study aimed to assess real-world post-marketing safety signals for this drug class. FAERS data from Q1 2018 to Q2 2025 were analyzed. CGRP antagonists included monoclonal antibodies (erenumab, fremanezumab, galcanezumab, eptinezumab) and small-molecule receptor antagonists (rimegepant, ubrogepant, atogepant). Disproportionality analyses were conducted using the reporting odds ratio (ROR) and information component (IC). The impact of age, sex, and weight on cAE was assessed. Time-to-onset analyses were also carried out. A total of 1806 cAE reports associated with CGRP antagonists were identified. Palpitations emerged as a consistent signal across all seven agents, suggesting a class effect. Monoclonal antibodies, particularly erenumab and fremanezumab, exhibited a broader spectrum of cAE signals, including coronary artery dissection, Prinzmetal angina, and postural orthostatic tachycardia syndrome. The results showed higher body weight was significantly associated with the cAE signal of disproportionate reporting (SDR) of erenumab (odds ratio [OR] 1.48, 95% CI 1.02-2.11, P = 0.034). Meanwhile, male sex was significantly associated with the cAE SDR of galcanezumab (OR 2.41, 95% CI 1.25-4.40, P = 0.006). Time-to-onset analyses indicated that most cAEs followed an early failure pattern, with the highest reporting intensity shortly after treatment initiation. Using FAERS, this pharmacovigilance study detected signals of disproportionate reporting of cardiac adverse events for most CGRP antagonists. These results are hypothesis-generating and reflect reporting patterns rather than incidence or relative risk; therefore, they should not be interpreted as evidence of causality or as patient-level cAE risk factors. Continued post-marketing surveillance and confirmatory pharmacoepidemiologic studies in well-defined populations are warranted.  CLINICAL TRIAL NUMBER: Not applicable.

Postural orthostatic tachycardia syndrome (POTS), characterized by a rise in heart rate of at least 30 beats per minute from supine to standing position without accompanying orthostatic hypotension, is one of the most common autonomic disorders with disabling cardiovascular and neurologic manifestations. Hormonal influence has been long recognized by the disorder predominantly affecting women of reproductive age, with frequent onset around menarche, exacerbation of symptoms before or during menses, and pregnancy being one of POTS triggers. Hormone replacement therapy (HRT) and menopause in women with POTS have not been studied, but issues surrounding HRT are highly relevant as women with POTS transition from reproductive age to menopause. Given a rising prevalence of POTS due to post-COVID onset and the US Food and Drug Administration recently removing the black box warning on estrogen-containing HRT formulations, informed decisions and risk assessments regarding HRT use in women with POTS are warranted. In this narrative review, existing studies on hormones and POTS and its common comorbidities are reviewed, and key points in decision-making on the use of HRT in women with POTS are discussed. In summary, for women with significant menopausal symptoms and/or exacerbation of POTS during the peri- or postmenopausal period, using some forms of HRT for treatment of menopausal symptoms may be considered, accounting for comorbidities, cardiovascular risk and other factors. Vaginal estrogen appears to be safe for most women while transdermal estrogen and micronized progesterone can be utilized for significant menopausal symptoms, although outcomes of their long-term use are unknown.

Patients with Postural Orthostatic Tachycardia Syndrome (POTS) and Orthostatic Intolerance (OI) commonly present with symptoms suggestive of Pelvic Venous Disorders (PeVD). The presence of PeVD may contribute to orthostatic symptoms in these patients due to venous obstruction with stasis and pooling. Iliac vein compression, a PeVD, has historically been treated with iliac venous stenting. The authors hypothesize that patients with POTS frequently have findings of PeVD, that venous outflow obstruction from iliac vein compression exacerbates POTS/OI symptomatology, and that treating PeVD improves POTS/OI symptoms. This paper reports results from two studies at two different institutions with a partially overlapping patient population. The first was a retrospective cross-sectional observational study of patients with POTS/OI who presented to Emory University Cardiology (Atlanta, GA, USA) from October 2019 to June 2023. We aimed to evaluate the prevalence of concurrent POTS/OI and PeVD using screening pelvic venous ultrasound, MR and/or CT, compared to venogram with intravascular ultrasound (IVUS). We secondarily evaluated the efficacy of each imaging modality in screening accuracy for PeVD. The second study was a retrospective review of medical records for 271 female patients with POTS/OI who received treatment of PeVD with iliac vein stenting from June 2019 to November 2024 at Minimally Invasive Procedure Specialists (Highlands Ranch, CO). The primary objective of this study was to explore quality of life (QoL) outcomes in female patients with POTS/OI before and after treatment. The secondary objective was to record the prevalence of associated pelvic pain, systemic symptoms, and response to therapy in this population. In the first cohort, 129 patients (84% cis female) with a diagnosis of POTS/OI and symptoms of PeVD were assessed by standard imaging for venous pathology. 107 patients (83%) had confirmed pelvic venous compression (iliac vein, renal vein, or both) or pelvic venous congestion on imaging with at least one screening modality. All screening modalities were relatively insensitive in detecting iliac venous compression compared with venography and IVUS. In the second cohort, following iliac vein stenting, significant improvements were seen in Orthostatic Hypotension Questionnaire (OHQ) composite scores at three months (p < 0·001) and at 12 months (p < 0·001). The OHQ Symptom Assessment (OHSA) and Daily Activities Scale (OHDAS) subscores, International Pelvic Pain Society (IPPS) score, Pelvic Congestion Syndrome (PCS) score, Pelvic Pain and Urgency/Frequency Symptom Scale (PUF) score, and Ancillary symptom score all demonstrated statistically significant decreases at three months that persisted at 12 months (all p < 0·001) as well. The high prevalence of PeVD in POTS/OI patients suggests an association between the two diagnoses. In the absence of expert clinical review, screening with US, CT, and MR may not be sufficient to rule out PeVD. Significant improvements were seen in QoL in patients with POTS/OI and PeVD who underwent iliac vein stenting. These results call for additional trials to examine the clinical and hemodynamic effects of venous stenting on POTS/OI symptomatology and associated systemic symptoms, including pelvic pain. These studies received no funding.